Circulating antineutrophil autoantibodies in a child with isolated centralnervous system vasculitis

An 8-year old girl with history of twisted neck and painful swelling on the left side of the neck was found to have malfunction of glossopharyngeal an d hypoglossal nerves on the left side. Magnetic resonance angiography revea led a giant aneurysm of the internal carotid artery surrounded by a widespr ead inflammatory tumor. Cerebral angiography disclosed a large, false aneur ysm with almost complete compression of the internal carotid artery. Circul ating antineutrophil cytoplasmic autoantibodies (titer 1:2560) and high lev els of antibodies against antiproteinase 3 were detectable. This observatio n indicates that these autoantibodies may be a diagnostic tool in children in whom an undiagnosed central nervous system inflammatory disease is prese nt.